Sturge – Weber syndrome (SWS) is a rare disorder that occur with a frequency of approximately 1 per 50,0001. It is a neurocutaneous syndrome, characterized by a facial vascular birthmark and neurological abnormalities. The hallmark is intracranial vascular angioma, most often involving the occipital and posterior parietal lobes, but it can also affect the other cortical regions2. An ipsilateral facial cutaneous vascular malformation (port wine nevus) usually affects the upper face3. Other clinical findings associated with SWS are seizures, glaucoma, hemiparesis, mental retardation and delayed developmental milestones4,5. This article reports a case of 8 years old boy who presented with weakness of right half of body since birth, mental retardation and delayed developmental milestones.Clinical examination revealed deep purple nevus on left lower face, and less power of left upper & lower limbs.X-ray skull showed calcification. C.T. Scan of brain revealed curvilinear calcification with focal atrophy.